A Case of Diffuse Panbronchiolitis Developing in Childhood / 소아과

Diffuse panbronchiolitis(DPB) is a chronic inflammatory airway disease predominantly affecting respiratory bronchioles, with distinct clinicopathological profiles. It was first described in 1966 by Yamanaka et al. The etiology of DPB is not yet clear, and the natural history of the disease is respiratory failure leading to cor pulmonale and ultimately death. But the long-term use of low-dose macrolide has proven to be highly effective in treating patients with DPB. Usual age at diagnosis is over 40. A few cases of DPB have been reported in Korea since 1992 but there have been no reports in children. We experienced a case of DPB in a 12-year-old girl. Therefore, we report the case with a brief review of the related literature.

A Case of Meningococcal Meningitis with Complement 9 Deficiency / 소아과

Meningococcal disease is not rarely associated with abnormalities of the complement system. We experienced a case of C9 deficiency with meningococcal meningitis from a 12-year-old girl. Identification of complement deficiency has implications for management, including family studies, prophylaxis, vaccination, and altered threshold for infection screening and treatment.

Clinico-epidemiologic Study of Mycoplasma pneumoniae Pneumonia(1993 through 2003) / 소아과

PURPOSE: We evaluated the epidemiologic and clinical characteristics of mycoplasma pneumonia. METHODS: A total of 559 medical records of children with mycoplasma pneumonia admitted to The Catholic University of Korea, Daejeon St. Mary's Hospital, were retrospectively analyzed. RESULTS: The mean annual number of cases was 51. There was a higher occurrence in autumn (September-November, 41.7%) and in winter(26.7%). Outbreaks of mycoplasma pneumonia were noted in 1993-94, 1997, 2001, and 2003. The age distribution showed a peak frequency of 5-6 years of age and 68.2 percent of patients were in 3-8 years of age. The male-to-female ratio was 1.2:1. In comparison between 1994 and 2003, there was a difference in age distribution with a peak frequency of 5-6 years of age in 1994, and of 3-4 years of age in 2003. There were outbreaks during autumn and winter in 1993-94, and during summer and autumn in 2003. CONCLUSION: Outbreaks of mycoplasma pneumonia occurred every 2-4 years in Daejeon in accordance with nationwide epidemics during 1993-2003. The peak incidence of age in the recent outbreak was younger than in the outbreak which occurred 10 years ago, and in outbreaks in Western countries.

Lipid Profile Changes During and after Kawasaki Disease

PURPOSE: We wanted to evaluate the serial lipid profiles in children with Kawasaki disease(KD) for a period of 1 year and the association of lipid profiles with the development of coronary artery lesions(CAL). METHODS: The subjects were 43 children with KD admitted at The Catholic University of Korea, Daejeon St. Mary's Hospital from January 2000 to December 2002. Serial sera were collected 4 times for 1 year from all patients; at admission(acute stage), 1-2 months, 6 months and 1 year after disease onset. The values of total cholesterol(TC), high-density lipoprotein cholesterol(HDL-C), low-density lipoprotein cholesterol(LDL-C) and triglyceride(TG) were measured by an automatic analyzer. The sera from 59 age- matched healthy children were used as controls. RESULTS: The values of TC, HDL-C, and LDL-C in KD patients in the acute stage were significantly lower than those at 1-2 months, at 6 months and at 1 year, and also lower than those in controls. HDL-C levels have a tendency to increase with a time elapsed. There was no significant difference in lipid profiles after the acute stage between the KD patients and control groups. In comparison between the group with CAL and the group without CAL, although the TC value in the acute stage was lower and TG value at 1 year was higher in the group with CAL than that in the group without CAL, there was no lipid profile with statistical significance between the groups. CONCLUSION: Our findings suggest that the altered lipid profile in the acute stage of KD may reflect the severity of inflammation in KD, and it recover within 2 months of disease onset. Alterations in lipid profile after the acute stage do not seem to be associated with the risk factor for premature atherosclerosis.

A Case of Atypical Kawasaki Disease with Autoimmune Hemolytic Anemia

Kawasaki disease(KD) is an acute febrile vasculitis with various clinical manifestations and the most common cause of acquired coronary arterial lesion in pediatric population. Occasionally, KD can be presented with some kind of unusual associations. of these, cases associated with autoimmune hemolytic anemia(AIHA) have been rarely reported worldwide. We report a case of KD associated with AIHA in the acute stage of disease and Parvovirus B19 infection, in a 4-month-old infant.

Epidemiologic Study of Aseptic Meningitis in Daejeon, Korea(1987 through 2003) / 소아과

PURPOSE: To evaluate the epidemiologic characteristics of aseptic meningitis in Daejeon, Korea from 1987 to 2003. METHODS: A total of 2201 medical records of children with aseptic meningitis admitted to the Catholic University of Korea, Daejeon St. Mary's Hospital, was retrospectively analyzed. RESULTS: The mean annual number of cases was 129, with a higher rate of occurrence in the summer season(May to August, 74.1%). Outbreaks of aseptic meningitis were noted in the years 1990, 1993, 1996, 1997, 2001, and 2002. The age distribution was relatively even, with higher incidences in the 4-7 years group and in the under one year old group. The male-to-female ratio was 2 : 1. Comparing the epidemics of 1997 and 2002, there were no marked differences in age distribution, except in the less than 1 year old age group(11.8% vs. 4.4%), nor in the monthly incidence. Neurologic complications were found in 0.7% of the patients. CONCLUSION: Outbreaks of aseptic meningitis occurred every one to three years in Daejeon, reflecting typical nationwide epidemics that occurred from 1987 to 2003. The incidence of disease in the less than 1 year age group during each epidemic may reflect the herd immunity of the maternal generation.

Inflammatory Processes in Kawasaki Disease Reach their Peak at the Sixth Day of Fever Onset: Laboratory Profiles According to Duration of Fever

We evaluated the inflammatory indices according to the fever duration in children with Kawasaki disease (KD), and determined duration when the inflammatory processes in KD reach their peak. Children with KD (n=152) were classified into 7 groups according to fever duration: at the third day or earlier (n=20), fourth (n=33), fifth (n=46), sixth (n=15), seventh (n=15), eighth (n=9), and at the ninth day or later after fever onset (n= 14). The levels of various laboratory indices were determined 3 times: before, 24 hr and 7 days after intravenous immunoglobulin administration (2 g/kg). WBC and neutrophil counts, and C-reactive protein level were the highest at the sixth day. Levels of hemoglobin, albumin, and high density lipoprotein cholestrol were the lowest at the sixth day. Although these indices were not significant statistically between groups, the indices showed either bell-shaped or U-shaped distribution of which peak or trench were at the sixth day. These findiugs showed that the inflammatory processes in KD reach peak on the sixth day of fever onset. This finding is important because a higher single-dose intravenous immunoglobulin treatment before the peak day may help reduce the coronary artery lesions in KD.

Clinical Significance of C-reactive Protein in Measles

PURPOSE: We evaluated clinical manifestations and laboratory findings in patients with measles according to C-reactive protein(CRP) concentration. METHODS: A retrospective analysis was performed using the medical records of patients with measles at The Catholic University of Korea, Daejeon St. Mary's Hospital from October 1999 to May 2000. We divided the patients with measles into four groups according to CRP level, i.e., those with below 5 mg/L(134 patients, negative group), those with 6-19 mg/L(85 patients), those with 20-49 mg/L(27 patients), and those over 50 mg/L(7 patients). We compared clinical and laboratory characteristics among these four groups. RESULTS: The mean CRP level of all patients was 11.1+/-7.5 mg/L. No statistical differences were present between the negative group and the 6-19 mg/L group or the 20-49 mg/L group in the duration of fever, hospitalization days, complications determined with longer hospitalization for more than eight days, white blood cell count, and incidence of hepatitis. Compared with the negative group, the over 50 mg/L group showed a longer duration of fever(4.7+/-1.7 vs 7.2+/-3.9 days), duration of hospitalization(5.4+/-1.4 vs 9.4+/-4.7 days), incidence of complications(5.2% vs 42.9%) and a higher mean level of WBC count(5,900+/-2,700/mm3 vs 12,700+/-6,700/mm3). With an increasing CRP level, there was a tendency for the duration of fever, complications and WBC count to increase. However the levels of liver enzymes(AST/ALT) were not associated with CRP level. CONCLUSION: A CRP level of over 50 mg/L in measles is associated with severity and complications.

The Relationship between Human Neutrophil Elastase and Coronary Arterial Dilatation in Kawasaki Disease

PURPOSE: Kawasaki disease is notorious for coronary arterial complication which is usually developed as a febrile disease in early childhood. Increased polymorphonucleus(PMN) cell levels in acute phases may be associated with the pathophysiology of Kawasaki disease. We studied the relationship between coronary arterial dilatation and elastase activity which was excreted from PMN cell and roles as an important factor for vasculitis. METHODS: Ten patients diagnosed with Kawasaki disease in Yonsei University Medical Center were examined between November, 2001 and January, 2002. In addition, 15 patients with other febrile diseases were also examined. Echocardiography was done in patients with Kawasaki disease on the first day of admission and four weeks after the onset of the disease. At each time, venous samples were drawn and separated into plasma and leukocytes. In patients with other febrile disease, samples were drawn on admission. Elastase activities in plasma and neutrophil extracts were measured. RESULTS: The significant increased plasma elastase activity, 6.19+/-0.74 U/mL, found in Kawasaki disease patients compared with the other febrile disease patients, 4.86+/-1.17 U/mL(P<0.05). And there was no significance between the above two diseases in terms of the elastase activity in neutrophil extracts. The relationship between initial elastase activity and the coronary arterial complication which was shown in subacute phase wasn't significant. CONCLUSION: Plasma elastase activity was increased in Kawasaki disease significantly, but the initial plasma elastase activity in the acute phase could not reflect the range of coronary arterial complication.

A Comparative Study of Measles According to Age

PURPOSE: We evaluated clinical manifestations and laboratory findings in patients with measles according to age distribution. METHODS: Retrospective analyses were performed using medical records of patients with measles admitted to The Catholic University of Korea, Daejon St. Mary's Hospital from October 2000 to May 2001. We divided the patients with measles into three age groups, i.e., those who were under two years of age(159 patients), those between 9-11 years of age(39 patients), and those older than 16 years of age who were hospitalized in the department of internal medicine(young adult group; 23 patients). We compared clinical and laboratory characteristics among these three groups. RESULTS: Almost all patients with measles were presented with fever, skin rash and cough. No statistical differences were present between the three groups in total fever duration, number of hospitalization days, complications determined with longer hospitalization for more than eight days, and positive values of anti-measles IgM. Patients under 2 years of age showed statistically higher levels of white bood cell and lymphocyte counts. However, neutropenia and lymphopenia were observed in all age groups compared with age-matched standard values. Campared with the other two age groups, the young adult group showed a higher mean level of liver enzymes(AST/ALT) and more patients with a level twice as high as the normal values. CONCLUSION: Clinical manifestations including complications according to age groups showed no significant differences in patients with measles. Hepatic involvement was more prevalent in the young adult group.

Laboratory Values in Patients with Kawasaki Disease after Intravenous Immunoglobulin: Comparison of Patients with Coronary Artery Lesions to those without Coronary Artery Lesions

PURPOSE: We evaluated the effects of intravenous immunoglobulin(IVIG) on level of laboratory parameters examined serially according to the existence of coronary artery lesions in children with Kawasaki disease. METHODS: Children with Kawasaki disease(n=63), treated with IVIG at a dose of 2.0 g/kg, were classified as a group with coronary artery lesions(CALs+ group, n=9) or a group without coronary artery lesions(CALs- group, n=54). Levels of various laboratory parameters were determined three times during admission; before, 24 hrs after and 7 days after IVIG administration. RESULTS: There were no significant differences in laboratory parameters performing at, before and 7 days after IVIG administration. However WBC and neutrophil counts, and CRP were significantly higher, and the level of albumin was significantly lower at 24 hrs after IVIG administration. CONCLUSION: Approximately 15% of patients with Kawasaki disease showed CALs in the acute stage. Kawasaki disease patients with CALs were associated with persistent elevated levels of inflammatory parameters including WBC count, neutrophil count and CRP examined 24 hours after IVIG administration.

Causative Organisms in Children with Bacterial Meningitis(1992-2002)

PURPOSE: This study was performed to assess the difference of organisms causing bacterial meningitis according to time. METHODS: We analyzed retrospectively 40 medical records of bacteriologically proven meningitis from 1992 to 2002. We divided them into two groups; neonate's group(14 cases), and children's group(26 cases). The results of the neonate's group were compared with those of previously reported articles in Korea, in 1970s-1980s. The causative agents of the children's group were analyzed according to the stage before and after the introduction of H. influenza type b(Hib) vaccine. RESULTS: In neonates, Group B streptococci(GBS) was the most common cause of bacterial meningitis. There was a trend in Korea that major causative agents of neonatal bacterial meningitis have changed from gram negative bacteria including E. coli to gram positive bacteria including GBS. In children, H. influenzae was isolated in six out of 11 cases(55%) in 1992-95, before the introduction of Hib vaccine, while two out of seven(29%) were isolated in 1999-2002, after the introduction of the Hib vaccine. CONCLUSION: Our study showed that the most common agent of neonatal bacterial meningitis was GBS. There was a trend that after the introduction of Hib vaccine, the incidence of H. influenza meningitis decreased in children.

A Case of Neonatal Submandibular Abscess

Suppurative sialadenitis is rare in the neonate and usually involves the parotid glands. Isolated suppurative submandibular sialadenitis in the neonatal population is extremely rare. There are only 11 cases found in the literature of suppurative submandibular sialadenitis occurring as an isolated lesion. We describe a case of isolated submandibular sialadenitis progressing to submandibular abscess requiring incision and drainage in a term neonate. Pus culture yielded methicillin-resistant Staphylococcus aureus (MRSA). A brief review of literature is included.

A Case of Malignant Extrarenal Rhabdoid Tumor of the Retroperitoneum / 대한소아혈액종양학회지

Malignant rhabdoid tumor is a clinically aggressive neoplasm that was initially described as a distinctive renal tumor of childhood. But among the malignant rhabdoid tumors, extrarenal rhabdoid tumor is rare. We report an extrarenal neoplasm histologically and ultrastructurally identical to renal rhabdoid tumor that arose in the retroperitoneum of a 4-month-old boy and presented as a right lower abdominal mass. The tumor had an aggressive clinical course despite multimodal therapeutic regimens, and the patient died with disseminated disease 12 months after diagnosis.

Predictive Values of High Dose Immune Globulin G or Oral Prednisone Therapy for the Splenectomy Response in Patients with Chronic Idiopathic Thrombocytopenic Purpura / 대한소아혈액종양학회지

PURPOSE: The aim of this study is to investigate the usefulness of responsiveness to high dose intravenous immune globulin G (IVIG) or oral prednisone therapy as preoperative predictors for splenectomy response in patients with chronic idiopathic thrombocytopenic purpura (ITP). METHODS: We reviewed retrospectively the charts of 23 patients who were admitted to Yonsei Medical Center, Wonju Christian Hospital, Ajou Medical College Hospital and Pochon CHA General Hospital under the diagnosis of chronic ITP and plenectomized from January 1990 to April 1999, below the age of 20. All of the patients had been treated with high dose IVIG and, or oral prednisone. The responses to the treatments were classified according to Berchtold and McMillan's criteria (1) complete response (CR) 50 103/L. RESULTS: Of 23 patients, 12 boys and 11 girls, the mean age at operation was 12.4 years (5.4~19.4 years), the mean duration from diagnosis to splenectomy was 47 months (6~173 months) and mean follow up was 33 months (3~95 months). Of the 6 patients with responses to oral prednisone, 5 had responses to splenectomy and of the 12 patients with reponses to IVIG, 10 had responses to splenectomy at 3 months. All of 14 patients with no response to oral prednisone and 4 patients with no response to IVIG were responsed to splenectomy at 3 months. Four patients relapsed during follow up and there was no serious complication following splenectomy. CONCLUSION: We concluded that a positive response to oral prednisone or IVIG may be associated with a positive response to subsequent splenectomy and splenectomy is an effective and safe treatment.